Multiple Sclerosis/Demyelinating Diseases

Question 1

temporary induction by heat or exercise of symptoms in patients with MS

Answer 1

Uhthoff Phenomenon

Question 2

flexion of the neck may induce a tingling, electric-like feeling down the shoulders and back, and less commonly the anterior thighs

Answer 2

Lhermitte sign

-it is probably attributable to an increase sensitivity of demyelinated axons to the stretch or pressure on the spinal cord induced by flexion

occurs in other conditions such as cervical spondylosis

Question 3

A temperature rise of ____ degree Celsius can block electrical transmission in thinly myelinated or demyelinated fibers

a. 0.5 C
b. 1 C
c. 5 C
d. 0.1 C

Answer 3

a. 0.5 C

page 941

Question 4

When an object such as a pendulum that is swinging perpendicular to the patient’s line of sight appears to move in a three dimensional circular path

Answer 4

pulfrich effect

Question 5

Components of Charcot Triad?

Answer 5

1. scanning speech
2. nystagmus
3. intention tremor

SIN

Question 6

rapidly progressive and highly malignant form of Multiple sclerosis presenting with a combination of cerebral, brainstem, and spinal manifestations evolving over weeks a few weeks

Answer 6

Marburg Variant/Tumefactive Multiple Sclerosis

Question 7

occurrence of alternating bands of destruction and preservation of myelin in roughly a series of concentric rings that represent alternating areas of myelin loss and preservation

Answer 7

Concentric sclerosis of Balo

Question 8

IgG index is considered a positive test of the result is greater than ______ of the total protein?

Answer 8

12%

Page 948

Question 9

Give four (4) conditions where oligoclonal bands may be positive:

Answer 9

1. Multiple sclerosis
2. Syphilis
3. Lyme disease
4. Subacute sclerosing Panencephalitis

Question 10

Most helpful ancillary examination in the diagnosis of MS

Answer 10

MRI

Page 948

Question 11

medication for MS that was initially shown to be efficacious but use was limited by risk of opportunistic infection with JC virus causing PML.

Answer 11

natalizumab

Question 12

The following are treatment options of Neuromyelitis optica, EXCEPT:

a. high dose IV corticosteroids
b. Interferon B
c. Plasma Exchange
d. azathioprine

Answer 12

except B

interferon B, natalizumab, fingolimod - may have deleterious effect on the relapse rate in NMO

Question 13

Difference of Reye’s syndrome vs post infectious encephalomyelitis? (based on labs)

Answer 13

Normal CSF

high serum concentration of liver enzymes and ammonia

Question 14

Most fulminant form of demyelinating disease - represents the severe end of the spectrum of ADEM

Answer 14

Acute Necrotizing Hemorrhagic Encephalomyelitis

Acute Hemorrhagic Leukoencephalitis of Weston Hurst

Question 15

Common locations of MS plaques (5)

Answer 15

infratentorial
juxtacortical
preiventricular
optic nerve
spinal cord

Question 16

What primarily differentiates NMO as a distinct entity from MS?*

Answer 16

Presence of specific anti-neural antibody

Question 17

Abnormalities of visual evoked responses have been found in how many percent of patients with clinical features of definite MS?

Answer 17

70%

page 949

Question 18

site of destruction of antibody in Devics Disease/NMO

Answer 18

astrocyte
or its myelin extension

(in contrast to MS - oligodendrocyte)

p 956